Congenital Scoliosis

Background 

• Epi 1-4% 
• Failure of neural axis 4-6w of development (mesenchymal anlage) 
• Progresses most rapidly in early years 

Etiology 

• Maternal 
  • DM 
  • Alcohol 
  • Valproic acid 
  • Hyperthermia 

Associated Conditions 

• 60% with associated 
• Cardiac, genitourinary, spinal cord 
• VACTERRL 
  • 38-55% 
  • Vertebral Malformation 
  • Anal atresia 
  • Cardiac 
  • Tracheal- Esophageal fistula 
  • Renal 
  • Radial anomaly 
  • Limb defects 
• Goldenhar 
• Jarco Levin 
  • Trunkal dwarfism 
  • Thoracic Insufficiency Syndrome 
• Klippel-Feil 
  • Fusion cervical vertebrae 
  • Short neck, low posterior hairline 
• Alagille syndrome 
  • Pulmonic stenosis, facial dysmorphism 
• Clubfoot 
• DDH 

Classification 

• Failure of formation (hemivertebrae) 
  • Fully segmented 
    • Disc on both sides 
  • Semi segmented 
    • Disc on one side, vert on another 
  • Unsegmented 
    • Vertebra on either side 
• Failure of segmentation (bars) 
  • Bilateral bony bars 
  • Unilateral bony bar 
    • High progression 
• Mixed 
  • Bar one side, hemi other side 

Evaluation 

• Renal US 
• Echocardiogram 
• MRI 
  • Chiari (syrinx), diastematomyelia (bony bar splits cord), tethered cord, intradural lipoma 
• Cervical XR 

Management 

• Non op 
• Operative 
  • Curve progression 
  • Anomaly has high likelihood of progression 
  • Refer to NSx for SC anomaly 

• Options 
  • In Situ Fusion 
    • Bar +/- hemi prior to major deformity 
    • A and P (or crankshaft) 
  • Convex Hemiepiphysiodesis 
    • Unilateral hemi (not a bar) 
      • Need growth remaining on other side 
    • Age <5 
    • Curve <40 
    • No truncal imbalance 
    • Technique 
      • Epiphysiodesis several spinal levels above and below the hemivertebra 
      • Guides gorwht back over 
      • Case for 6 months 
      • 15 degrees  ofrection 
  • Hemivert Excision 
    • Age <5 
    • Curve >40 
    • Truncal imbalance 
  • Correction and Fusion/ Osteotomies and PSIF 
    • Older 
    • Osteotomies for rigid 
  • Distraction based growing rods 
    • Indications 
      • Prevent thoracic insufficiency syndrome 
    • Technique 
      • Q 6 months adjustments  
    • Instrumentation 
      • Pedicle Based Instrumentation 
      • Rib based instrumentation 
        • VEPTR: Vertical Expandable Prosthetic Titanium Rib 

Complications 

• Crankshaft 
• Short stature 
• Neurologic injury 

• What are 3 associated conditions? 
  • Cardiac 
  • Genitourinary 
  • Spinal cord (30-40%) 
    • Tethered cord 
    • Diastatemelia 
      • No surgery unless myelopathic 
    • syringomyelia 
• What morphologies fastest too slowest progression has fastest progression 
  • Bar with contralateral hemi 5-10 per yaer 
  • Unibar 5 
  • Fully seg hemi 2-5 
  • Unincarcerated Hemi 
  • Incarcerated hemi 
  • Unsegmented hemi 
  • Block 
• Tests to order? 
  • Renal US 
  • Echo 
  • MRI 
  • C Spine XR